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ORPHA:97287

Bronchial neuroendocrine tumor

high confidenceDisorder

Also known as: Bronchial NET

Publications

184

61.3th percentile

Trials

1

Interventional, condition-specific

Researchers

1,232

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare neuroendocrine neoplasm characterized by origin from pulmonary neuroendocrine cells and ranging from low-grade typical carcinoid and intermediate-grade atypical carcinoid to high-grade large-cell neuroendocrine carcinoma and small-cell carcinoma. Two thirds of the tumors are located in the major bronchi, with a predilection for the right lung, in particular the middle lobe. Most patients with central bronchial tumors present with hemoptysis, cough, recurrent pulmonary infections, fever, chest discomfort, and unilateral wheezing, while peripheral carcinoids are usually discovered only incidentally. Carcinoid syndrome or Cushing syndrome are very rare. The tumors may be part of multiple endocrine neoplasia type 1.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (7)

bronchial NET · bronchus NET · bronchus neuroendocrine neoplasm · bronchus neuroendocrine tumor · bronchus neuroendocrine tumor, well differentiated, low or intermediate grade · bronchus neuroendocrine tumour · neuroendocrine neoplasm of bronchus

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

3/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    184 matched papers (133 in last 10 years) Source

  3. Phenotype characterisedPresent

    31 HPO annotations (e.g. Increased serum serotonin; Facial telangiectasia; Bowel urgency) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    1 matched on ClinicalTrials.gov (1 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

31

Associated phenotypes · MONDO:0019963

  • Increased serum serotonin
  • Facial telangiectasia
  • Bowel urgency
  • Poor appetite
  • Dermatological manifestations of systemic disorders

Showing 5 of 31 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

4

Drugs / clinical candidates · MONDO_0019963

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

184

184 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

184 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

133 in the last 10 years · high confidence · 61.3th percentile (publications denominator)

Phrase hits: 184 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,232

Distinct author names in 184 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Faggiano A6 papers · 2025

    Endocrinology, Department of Experimental Medicine, "Sapienza", University of Rome, Rome, Italy. antiongiulio.faggiano@uniroma1.it.

    Papers in Europe PMC
  2. 02
    Colao A5 papers · 2025

    Endocrinology, Department of Clinical Medicine and Surgery, "Federico II" University of Napoli, Napoli, Italy.

    Papers in Europe PMC
  3. 03
    de Herder WW5 papers · 2025

    Department of Internal Medicine, Section of Endocrinology, ENETS Center of Excellence, Erasmus MC and Erasmus Cancer Institute, Rotterdam, The Netherlands.

    Papers in Europe PMC
  4. 04
    Hofland J5 papers · 2025

    Department of Internal Medicine, Section of Endocrinology, ENETS Center of Excellence, Erasmus MC and Erasmus Cancer Institute, Rotterdam, The Netherlands.

    Papers in Europe PMC
  5. 05
    Brabander T4 papers · 2025

    Department of Radiology & Nuclear Medicine, ENETS Center of Excellence, Erasmus MC and Erasmus Cancer Institute, Rotterdam, The Netherlands.

    Papers in Europe PMC
  6. 06
    Liu Y4 papers · 2023

    Department of Radiation Oncology, The Affiliated Tumor Hospital of Zhengzhou University, Zhengzhou, China.

    Papers in Europe PMC
  7. 07
    Bodei L3 papers · 2020

    Molecular Imaging and Therapy Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

    Papers in Europe PMC
  8. 08
    Capdevila J3 papers · 2022

    Vall d’Hebron University Hospital, Vall d’Hebron Institute of Oncology (VHIO), Barcelona, Spain

    Papers in Europe PMC
  9. 09
    Caplin M3 papers · 2026

    Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

    Papers in Europe PMC
  10. 10
    Chan JA3 papers · 2020

    Dana-Farber Cancer InstituteBoston, Massachusetts, USA.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; 1 currently recruiting in our sample.

Data as of 11 September 2026

1 interventional trial — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 80.1th percentile).

high confidence · 80.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

1 interventional trials matched after quoted-phrase search and title/condition post-filter.

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 7 · after dedupe 7 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 7 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (7)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Bronchial neuroendocrine tumor — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Bronchial neuroendocrine tumor" OR "Bronchial NET" OR "bronchus NET" OR "bronchus neuroendocrine neoplasm" OR "bronchus neuroendocrine tumor" OR "bronchus neuroendocrine tumor, well differentiated, low or intermediate grade" OR "bronchus neuroendocrine tumour" OR "neuroendocrine neoplasm of bronchus" OR "neuroendocrine neoplasm of the bronchus"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Bronchial neuroendocrine tumor" OR "Bronchial NET" OR "bronchus NET" OR "bronchus neuroendocrine neoplasm" OR "bronchus neuroendocrine tumor" OR "bronchus neuroendocrine tumor, well differentiated, low or intermediate grade" OR "bronchus neuroendocrine tumour" OR "neuroendocrine neoplasm of bronchus" OR "neuroendocrine neoplasm of the bronchus"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 1 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T05:08:09.581Z